Retroperitoneal fibrosis: case series of five patients and review of the literature / Fibrose retroperitoneal: série de cinco casos e revisão da literatura

ABSTRACT Chronic periaortitis (CP) is an umbrella term used to describe a group of nosologically allied conditions that include idiopathic retroperitoneal fibrosis (Ormond's disease), inflammatory abdominal aortic aneurysm, and perianeurysmal retroperitoneal fibrosis. Retroperitoneal fibrosis encompasses a range of diseases characterized by the presence of a fibro-inflammatory tissue, which usually surrounds the abdominal aorta and the iliac arteries and extends into the retroperitoneum to envelop neighboring structures-ureters. Retroperitoneal fibrosis is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Here we describe a 5 years follow up (2006-2011) of 5 patients admitted to our hospital with symptoms, laboratory, imaging and pathologic finding compatible with retroperitoneal fibrosis. We review our clinical course of our patient with respect to the literature.

RESUMO Periaortite crônica (PC) é um termo genérico usado para descrever um grupo de condições nosologicamente ligadas que incluem a fibrose idiopática retroperitoneal (doença de Ormond), o aneurisma da aorta abdominal inflamatório e a fibrose retroperitoneal perianeurismática. O termo fibrose retroperitoneal engloba uma gama de doenças que se caracterizam pela presença de um tecido fibroinflamatório que geralmente envolve a aorta abdominal e as artérias ilíacas, se estende ao retroperitôneo e envolve estruturas ureterais vizinhas. A fibrose retroperitoneal geralmente é idiopática, mas pode também ser secundária ao uso de determinados fármacos, doenças malignas, infecções e cirurgia. Este estudo descreve o seguimento por cinco anos (2006-2011) de cinco pacientes internados em nosso hospital que apresentavam sintomas e achados laboratoriais, de imagem e patológicos compatíveis com a fibrose retroperitoneal. Revisou-se a evolução clínica dos pacientes, que foi comparada com os achados da literatura.

Fibrosis retroperitoneal: presentación de un caso y revisión del tema / Retroperitoneal fibrosis: case report and literature review

Introducción: la primera descripción de obstrucción ureteral extrínseca por un proceso de fibrosis retroperitoneal se efectuó en 1905. En poco más de un siglo sólo se han reportado unos 800 casos de esta patología. Caso clínico: se reporta el caso de un paciente femenino de 55 años, que cursa con dolor abdominal difuso de larga evolución, acompañado de náusea, vómito, fiebre de 38°C e hipertensión de 160/100 mmHg. Se realiza tomografía axial computarizada observándose masa retroperitoneal que produce obstrucción de uréteres. Se realiza laparotomía exploradora con toma de biopsia y liberación de uréteres. El análisis histopatológico demuestra la presencia de fibrosis retroperitoneal, una entidad patológica poco frecuente, cuyo diagnóstico requiere un alto índice de sospecha en base a los hallazgos clínicos, de laboratorio y de gabinete. Revisión de la literatura: no se han formulado definiciones claras de los diferentes trastornos que se incluyen en el espectro de la fibrosis retroperitoneal, debido a lo infrecuente de esta enfermedad. Por esta razón hoy se carece de criterios diagnósticos y de una clasificación coherente de las diferentes formas que puede adoptar la enfermedad. Sin embargo, ante la sospecha de una fibrosis retroperitoneal se debe distinguir entre una forma idiopática y una secundaria, por las diferentes implicaciones para el tratamiento. Conclusión: la fibrosis retroperitoneal es un diagnóstico diferencial ante la presencia de dolor abdominal difuso asociado a síntomas de compresión ureteral o de grandes vasos.

Introduction. The first description of extrinsic uretheral obstruction by retroperitoneal fibrosis occurred in 1905. In little more than a century, about 800 cases of this disease have been reported. Case description. We report the case of a female 55 year-old patient who presents with diffuse abdominal pain of long duration, nausea, vomiting, fever of 38°C and hypertension of 160/100 mmHg. A CT scan is performed that shows a retroperitoneal mass that obstructs the urethers. Exploratory laparotomy was performed, urethers were released and biopsy was taken. Pathology analysis showed the presence of retroperitoneal fibrosis, a rare pathological entity whose diagnosis requires a high index of suspicion based on clinical, imaging and laboratory workup. Literature review. There are no clear definitions of the variety of disorders that are included in the spectrum of retroperitoneal fibrosis, due to the rarity of this condition. Consequently, we lack diagnostic criteria and a consistent classification of the different forms that it may adopt. However, when there is suspicion of retroperitoneal fibrosis, the first step is to establish whether it is idiopathic or secondary, as there will be treatment implications. Conclusion. Retroperitoneal fibrosis should be considered in the differential diagnosis whenever diffuse abdominal pain is associated with uretheral or great vessels compression.

Tratamento associado (clínico-cirúrgico) da fibrose retroperitoneal idiopática / Associated treatment (medical and surgical) of idiopathic retroperitoneal fibrosis

Os autores descrevem caso de paciente com fibrose retroperitoneal idiopática e revisão da patologia. Esta rara doença de etiologia incerta caracteriza-se por inflamação crônica do retroperitôneo, ocasionalmente aprisionando e obstruindo estruturas, notavelmente os ureteres. Trata-se de um paciente do sexo masculino, 45 anos, com dor em flanco esquerdo irradiada para testículo, em cólica, com piora progressiva. Feito o diagnóstico de fibrose retroperitoneal com compressão ureteral à esquerda (tomografia computadorizada do abdome), o paciente foi submetido a desobstrução ureteral com colocação de cateter duplo “J” e, posteriormente, a tratamento clínico com prednisona. Não houve regressão da massa fibrótica, mas melhora clínica quanto à dor e à ausência de novo episódio de obstrução ureteral. Os autores ressaltam a necessidade de suspeição da patologia descrita, mesmo sendo rara, pela possibilidade de complicações graves, se seu diagnóstico não for realizado e o tratamento não for instituído no tempo ideal

A case report of a patient with idiopathic retroperitoneal fibrosis (RPF) is described and the condition is reviewed as well. RPF is a rare disease of unclear etiology characterized by chronic retroperitoneal inflammation, which can entrap and obstruct retroperitoneal structures, notably ureters. A 45-year-old male patient presented with colic-likeleft flank pain irradiating to the testicle and progressively worsening. Retroperitoneal fibrosis with left ureteral compression was diagnosed in a abdominal computed tomography scan and the patient was submitted to reteral unblocking with insertion of a double-J catheter and then to clinical treatment with prednisone. Although there was no regression of the fibrotic mass, pain clinically relieved and no new ureteral obstruction episodes were seen. This case illustrates the importance of suspecting the above described condition. Although rare, RPF can potentially cause severe complications if it is not diagnosed and treated timely

Laparoscopic treatment of retroperitoneal fibrosis: report of two cases and review of the literature

OBJECTIVES: We present the results of treatment by laparoscopy of two patients with retroperitoneal fibrosis and review the literature since 1992, when the first case of this disease that was treated using laparoscopy was published. We also discuss the contemporary alternatives of clinical treatment with corticosteroids and tamoxifen. CASE REPORT: Two female patients, one with idiopathic retroperitoneal fibrosis, and other with retroperitoneal fibrosis associated with Riedel's thyroiditis, were treated using laparoscopic surgery. Both cases had bilateral pelvic ureteral obstruction and were treated using the same technique: transperitoneal laparoscopy, medial mobilization of both colons, liberation of both ureters from the fibrosis, and intraperitonealisation of the ureters. Double-J catheters were inserted before the operations and removed 3 weeks after the procedures. The first patient underwent intraperitonealisation of both ureters in a single procedure. The other had 2 different surgical procedures because of technical difficulties during the first operation. Both patients were followed for more than 1 year and recovered completely from the renal insufficiency. One of them still has occasional vague lumbar pain. There were no abnormalities in the intravenous pyelography in either case. CONCLUSIONS: Surgical correction of retroperitoneal fibrosis, when indicated, should be attempted using laparoscopy. If possible, bilateral ureterolysis and intraperitonealisation of both ureters should be performed in the same operation

Embarazo abdominal genuino con feto vivo de término / Real abdominal pregnancy with alive term fetus

Se presenta un caso de embarazo abdominal, con feto maduro, como hallazgo de cirugía electiva en un caso de RCIU más oligohidroamnios severo y presentación podálica. Se realiza extracción fetal y extracción placentaria. Se obtiene un recién nacido sano. La evolución postoperatoria es favorable hasta el alta. Presenta complicación de diagnóstico tardío consistente en fibrosis retroperitoneal que produce hidroureteronefrosis izquierda que termina finalmente en nefrectomía

Fibrosis retroperitoneal idiopática (FRI): presentación de un caso clínico y revisión de la literatura / Idiopatic retroperitoneal fibrosis (IRF): a case report and literature review

Se describe el caso de un paciente de sexo masculino, 44 años con una fibrosis retroperitoneal idiopática derecha. El paciente se presentó con lumbalgia de 10 años evolución, cólico renal derecho reciente y masa presacra palpable por tacto. La UIV mostró hidronefrosis derecha. Se practicó ureterolisis, reimplantante de uréter derecho y bypass de arteria ilíaca común a ilíaca externa. El paciente se mantiene sin tratamiento anticoagulante y al año, la UIV es normal

Fibrose retroperitoneal: relato de caso / Retroperitoneal fibrosis: report of a case

Os autores relatam um caso de fibrose retroperitoneal diagnosticada por laparotomia exploradora a qual havia sido indicada por uma hipótese diagnóstica de aneurisma de aorta fissurado. Dada a raridade da doença, fazem o presente relato e revisam a literatura

Autotransplante renal no tratamento da fibrose retroperitoneal: relato de caso / Renal autotransplantation in the treatment of idiopathic retroperitoneal fibrosis: case report

A fibrose retroperitoneal idiopática é doença de etiología desconhecida, caracterizando-se por densa placa retroperitoneal que envolve um ou ambos ureteres, causando hidronefrose e impedindo a funçäo renal. A doença primária ou recorrente pode produzir dano ureteral extenso, resultando nas limitas opçöes cirúrgicas para a soluçäo do problema. Säo revisados 625 casos de fibrose retroperitoneal publicados na literatura. Säo descritos os fatores etiológicos, a idade, o sexo, os sinais, os sintomas, os exames complementares e o tratamento. É apresentado um caso tratados através de autotransplante renal